Bayley-Pinneau method of height prediction in girls with central precocious puberty: correlation with adult height.

One of the sequelae of idiopathic central precocious puberty (ICPP) can be short adult stature. In this retrospective study adult height was normal in 90% of girls with untreated ICPP (mean, 161.4 +/- 7.7 cm). The height prediction made at the time of initial examination and the height age correlated with adult height. Therefore the initial height prediction can be useful in identifying those girls with ICPP at risk for short stature.

Preservation of physiological growth hormone (GH) secretion in idiopathic short stature after recombinant GH therapy.

This study was undertaken to assess the recovery of spontaneous GH secretion 48 h after the cessation of GH therapy in children with idiopathic short stature treated with recombinant DNA-generated human GH (rhGH-M). Eleven prepubertal children with GH responses of 10.0 ng/mL or more after provocation were divided into therapeutic (n = 7) and control (n = 4) groups. GH was sampled every 20 min for 24 h in six treated and three control patients. One treated and one control patient had 12-h overnight studies because of their weight. The sampling studies were carried out before GH therapy was initiated and 48 h after rhGH was discontinued after 12 months of therapy. Three patients in the treated group also underwent a 24-h study at the 6 month time point. The treated group started treatment with rhGH (0.1 mg/kg), given three times a week. The results showed that pre- and posttreatment GH secretory profiles were comparable with respect to the number of peaks, mean concentrations, peak amplitude, and secretory rate. At the 6 month point, the mean GH and peak GH amplitude (n = 3) were greater than the means of the treatment group (n = 7) at the 0 and 12 month points, but the difference was not statistically significant. Somatomedin-C rose in the treated group from 0.42 +/- 0.1 to 1.25 +/- 0.3 U/mL (mean +/- SD; P less than 0.01). In the control group it rose from 0.56 +/- 0.1 to 1.16 +/- 0.8 U/mL (mean +/- SD; P greater than 0.05) because one patient entered puberty in the 12-month period of observation; his somatomedin-C level rose from 0.72 to 2.5 U/mL. We conclude that exogenous GH therapy does not interfere with the maintenance of endogenous pulsatile secretion of GH. These data show that exogenous GH therapy does not interfere with the maintenance of endogenous pulsatile secretion of GH and provide evidence for the resilience of the GH secretory system in the growing child.

Pubertal growth in patients with androgen insensitivity: indirect evidence for the importance of estrogens in pubertal growth of girls.

Spontaneous pubertal growth was studied in eight patients with the syndrome of androgen insensitivity to obtain information on the growth-promoting action of estrogens. In one additional patient (who had a gonadectomy before puberty), the effect of exogenous estrogens was studied. Mean age at peak height velocity (12.7 years) was closer to that in normal girls than to that in normal boys. Mean peak height velocity (7.4 cm/yr) was as in normal girls (7.3 cm/yr), but was lower than in normal boys (9.3 cm/yr). Bone age corresponded better to male standards. Mean adult height (172.3 cm) was lower than in normal men (-0.6 SD), but higher than in normal women (+1.4 SD). In the patient who had a gonadectomy, estrogen replacement caused a higher peak height velocity (12 cm/yr), but lower adult height (160.5 cm) than in the patients with intact gonads who received no treatments. We conclude that in normal girls, the pubertal growth spurt also results from the action of estrogens rather than of adrenal androgens. To ensure normal pubertal growth, physiologic estrogen replacement in hypogonadal females should be started at a bone age of about 11 years, and should not be delayed in the hope of achieving a greater mature height.

Compensatory growth after HLA-typing revelation of incorrect diagnosis of 21-hydroxylase deficiency.

A 9 1/2 year old boy had been treated for complete 21-hydroxylase deficiency from infancy. That diagnosis was excluded by HLA-typing of family members who wished to know their carrier status. Withdrawal of replacement therapy was very well tolerated. The patient showed distinct compensatory growth and for the first time was free of crises of adrenal insufficiency. This instance demonstrates an unexpected application of HLA-typing. It also shows that an amount of hydrocortisone too small to give any clinical evidence of overdose can impede growth. Furthermore, it provides reassurance that adrenocortical function can recover completely after many years of treatment with exogenous steroids.

Precocious puberty of cerebral origin.

Eighty-two cases of precocious puberty of cerebral origin were reviewed. All shared as a common factor the distortion, compression, or destruction of diencephalic structures. Attempts were made to parallel basic research findings with those derived from human pathology, hoping to gain further insight into the physiopathology of precocious puberty of cerebral origin.

Effects of isolation, handling and restraint on growth of rats.

The effects of 3 environmental conditions on physical growth and skeletal maturation were assessed in pair-fed rats. Rats subjected to restraint from ages 28 to 80 days weighed less and were shorter than pair-fed like-sex controls; skeletal maturation was unaffected. The observations cannot be attributed to handling, as those animals were heavier than their controls, or to isolation, which was found to be without effect on any of the measurements. The adverse effects of restraint on physical growth are attributed to increased adrenal cortical activity, reflected in increased adrenal weight.

Effects of neonatal stunting on the development of rats: early and late effects of neonatal cortisone on physical growth and skeletal maturation.

Cortisone acetate, 1.25 mg, was given im to each pup of eight eight-pup litters; saline was given to each pup of eight litters. At 21 days body weight, stem length, and length of long bones was less in the treated animals (P less than 0.001). The number of ossification centers was greater in the treated animals (P less than 0.05). Brain weight was less in the treated animals (P less than 0.001). For 84-day-old animals body weight (P less than 0.02) and length of most long bones (P less than 0.05) were less in the treated females. Body weight (P less than 0.01), stem length (P less than 0.01) and some bones (P less than 0.02) were smaller in the treated males. There was no difference in the number of epiphyseal fusions. The brains of the treated males weighed less than those of the controls (P less than 0.01). The effect on linear growth is in conformity with observations in children but the accelerated skeletal maturation was unexpected. The effect on skeletal maturation was less persistent than that on bone length.

Endocrine tests in phenotypic children with bilateral impalpable testes can reliably predict "congenital" anorchism.

Congenital anorchism is a rare condition. Bilateral impa-pable undescended testes are relatively common by comparison. Surgical exploration has been regarded as the final arbiter between anorchism and bilateral cryptorchism. Exploration has not proved completely reliable in making this differentiation. Endocrine studies, particularly the human chorionic gonadotropin (HCG) stimulation test together with measurements of basal plasma gonadotropins, can reliably exclude "functioning" testicular tissue. Eleven fully evaluated and operated cases support this contention. In the specific clinical setting of a normal phenotypic male child with a 46XY karyotype and no müllerian structures palpable on rectal examination, nonfunctioning testes on endocrine testing means congenital anorchism and surgical confirmation is unnecessary. In contradistinction, a positive HCG test would appear to mandate through and extensive surgical exploration.

Monozygotic twins discordant for sex.

A pair of monozygotic, adolescent twins is discordant for sex. The phenotypic female twin has chromosome constitution of 46, XY/45, X. She displays many signs of Turner's syndrome, including typical facies, webbed neck, malformed left kidney, high plasma gonadotropins, and streak ovaries. However, her height is 154 cm which exceeds the height usually reported in Turner's syndrome. The male twin has a karyotype of 46, XY and normal sexual development. Only two other reports of pairs of monozygotic twins of opposite sex have been published.